Evaluation, diagnosis and surveillance of renal masses in the setting of VHL disease
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INVITED REVIEW
Evaluation, diagnosis and surveillance of renal masses in the setting of VHL disease Jad Chahoud1 · Melissa McGettigan2 · Nainesh Parikh2 · Ronald S. Boris3 · Othon Iliopoulos4 · W. Kimryn Rathmell5 · Anthony B. Daniels6 · Eric Jonasch7 · Philippe E. Spiess1 on behalf of International VHL Surveillance Guidelines Consortium-Renal Committee Received: 18 June 2020 / Accepted: 3 September 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract This brief report focuses on the evaluation and diagnosis of clinically localized renal masses in children and adults with Von Hippel–Lindau (VHL) disease. Counseling considerations pertinent to the urologists, medical oncologists, and multidisciplinary teams involved in the care of these patients are addressed. As practice patterns regarding the evaluation and management of VHL tumors can vary considerably, this report aims to provide guidance on some of the controversies associated with the diagnostic evaluation and initial management of localized renal masses in VHL patients. Keywords Renal mass · Von Hippel–Lindau · VHL · Renal cell carcinoma · RCC
Background Von Hippel–Lindau (VHL) disease is a rare syndrome associated with multiple benign and malignant tumors, including renal cell carcinoma (RCC) [1–4]. The evaluation and management of these renal masses is of high importance, as * Jad Chahoud [email protected] 1
Department of Genitourinary Oncology, H. Lee Moffitt Cancer Center, 12902 Magnolia Dr., Tampa, FL 33612, USA
2
Department of Diagnostic Imaging and Interventional Radiology, H. Lee Moffitt Cancer Center, 12902 Magnolia Dr., Tampa, FL 33612, USA
3
Department of Urology, Indiana University School of Medicine, Indianapolis, IN, USA
4
Center for Cancer Research, Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, MA 02139, USA
5
Department of Medicine, Vanderbilt University Medical Center, Nashville, TN 37232, USA
6
Division of Ocular Oncology and Pathology, Department of Ophthalmology and Visual Sciences, Vanderbilt-Ingram Cancer Center, Vanderbilt University Medical Center, Nashville, TN, USA
7
Department of Genitourinary Medical Oncology, UT MD Anderson Cancer Center, 1515 Holcombe Ave, Houston, TX 77030, USA
these tumors decrease the life expectancy of VHL patients and impart significant distress to both patients and their families [5]. The impact of these renal masses on patient mortality is driven by end-stage renal failure secondary to surgical resections or the development of metastatic RCC. Therefore, a well-described approach that incorporates timely detection of renal masses and balances the appropriate timing for active surveillance and surgical resection is highly important for treating patients with VHL disease. Renal masses present at an early age as multicentric renal cysts of various complexity. These masses are found in approximately 42–63% of patients; the earliest age at presentation of this disease is noted to be 16 years, with a mean age of 37 years
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